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Published on: December 6, 2016
Achondroplasia and obstructive sleep apnea: surgical outcomes and comparison to general population
Claudio Gomez Ascencio1, Anna Wani1, Ron B Mitchell1
1University of Texas Southwestern Medical Center, Dallas, Texas.
Insights
Children with achondroplasia and obstructive sleep apnea (OSA) experience earlier onset and benefit from adenotonsillectomy for moderate to severe OSA. Surgical interventions like cervicomedullary decompression also improved apnea-hypopnea index.
Area of Science:
- Pediatric Sleep Medicine
- Genetics and Rare Diseases
- Surgical Outcomes
Background:
- Achondroplasia is a genetic disorder characterized by disproportionate dwarfism.
- Obstructive sleep apnea (OSA) is a common comorbidity in children with achondroplasia.
- Understanding the demographics and treatment efficacy in this population is crucial.
Purpose of the Study:
- To compare the demographics of children with achondroplasia and OSA to the general pediatric OSA population.
- To evaluate the surgical treatment outcomes for children with achondroplasia and OSA.
- To identify differences in age of onset and treatment response.
Main Methods:
- Retrospective chart review of 22 children with achondroplasia and OSA and 141 controls with OSA.
- Analysis of polysomnography parameters before and after surgical interventions.
- Comparison of baseline characteristics between the achondroplasia and control groups.
Main Results:
- Children with achondroplasia and OSA presented at a significantly younger age (3.1 years) for adenotonsillectomy compared to controls (6.8 years).
- Adenotonsillectomy in achondroplasia patients with moderate to severe OSA led to improvements in obstructive apnea-hypopnea index and oxygen saturation.
- Adenoidectomy alone did not significantly alter OSA, while cervicomedullary decompression improved the obstructive apnea-hypopnea index.
Conclusions:
- Patients with achondroplasia and OSA exhibit an earlier age of onset compared to their non-achondroplasia counterparts.
- Adenotonsillectomy is an effective treatment for moderate to severe OSA in children with achondroplasia.
- Cervicomedullary decompression offers a potential benefit for improving OSA in this population.
Study Objectives:
The objective was to compare demographics between children with achondroplasia and obstructive sleep apnea (OSA) with the general pediatric population with OSA, as well as present treatment outcomes for children with achondroplasia.
Methods:
Retrospective chart review of 22 children with achondroplasia and OSA and 141 children with OSA without achondroplasia. Parameters from polysomnography were analyzed. Values before and after surgery were compared for the achondroplasia group, while baseline values were compared between the control group and achondroplasia group.
Results:
Preintervention in the adenotonsillectomy achondroplasia group, 0 children had mild OSA, 1 had moderate OSA, and 10 had severe OSA. In the comparative group 16 had mild OSA, 11 had moderate OSA, and 114 had severe OSA. The achondroplasia population had a much younger age at adenotonsillectomy compared to the control population-3.1 vs 6.8 years. When comparing baseline data in the achondroplasia population with respective adenotonsillectomy outcomes, obstructive apnea-hypopnea index and oxygen saturation percentage were improved. For the adenoidectomy group, there were no significant changes in OSA after surgery. For the cervicomedullary decompression group, there was a decrease in obstructive apnea-hypopnea index after surgery.
Conclusions:
Patients with achondroplasia and OSA have an earlier age of onset compared to children without achondroplasia. For these patients with moderate to severe OSA, treatment with adenotonsillectomy leads to significant improvement. Treatment with adenoidectomy showed no significant change in OSA. Cervicomedullary decompression also led to an improvement in obstructive apnea-hypopnea index.
Citation:
Ascencio CG, Wani A, Mitchell RB. Achondroplasia and obstructive sleep apnea: surgical outcomes and comparison to general population. J Clin Sleep Med. 2025;21(5):883-890.
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